Abstract
Background
The California Encephalitis Project (CEP) is a program designed to determine causes of encephalitis. We sought to determine whether there are any distinguishing characteristics of patients with encephalitis who develop refractory status epilepticus from those who do not.
Methods
Data from all patients in the CEP were retrospectively reviewed and analyzed. Diagnostic testing was performed for a panel of infectious agents and medical information collected using a standardized form. Encephalitis patients were subdivided into three categories: (i) patients with status epilepticus unresponsive to standard antiepileptic therapy who required general anesthetic coma for management (Group I), (ii) patients with seizures or status epilepticus responsive to standard antiepileptic therapy (Group II), and (iii) patients without seizures (Group III). Supplementary information was requested on Group I patients.
Results
Of 1,151 patients; 43 (4%) were classified as Group I, 459 (40%) as Group II, and 649 (56%) as Group III. Compared to Groups II and III, Group I patients were younger (median age = 10.0 years), more likely to have fever (93%), prodromal respiratory (57%) or gastrointestinal illness (64%), and less likely to have CSF pleocytosis (47%) or abnormal neuroimaging (16%). A causative infectious agent was verified in three of the Group I patients; and a putative agent in nine others. Supplementary information on Group I revealed that 28% died within 2 years and 56% were neurologically impaired or undergoing rehabilitation.
Conclusions
Encephalitis and refractory status epilepticus occur most commonly in the pediatric age group, an infectious etiology is usually not established, and outcomes are generally poor.
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Acknowledgments
We thank the clinicians who referred patients to the CEP. We thank Fred Schuster, David Schnurr, Karen Bloch, and Lisa Bateman for their careful review of the manuscript. We also gratefully acknowledge the laboratory staff in the CDHS VRDL and Microbial Disease Laboratory for performing the diagnostic testing. We confirm that we have read the journal’s position on issues involved in ethical publication and affirm that this report is consistent with those guidelines. Financial support: Centers for Disease Control and Prevention Emerging Infections Program (U50/CCU915546–10). M. Solbrig is supported by the National Institute of Neurological Disorders and Stroke (NINDS) grant NS042307 and D. Lowenstein by NINDS grants NS053998, NS056975, and U54RR023566.
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Appendix A: California Encephalitis Project Case History Form
Appendix A: California Encephalitis Project Case History Form
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Glaser, C.A., Gilliam, S., Honarmand, S. et al. Refractory Status Epilepticus in Suspect Encephalitis. Neurocrit Care 9, 74–82 (2008). https://doi.org/10.1007/s12028-007-9042-y
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DOI: https://doi.org/10.1007/s12028-007-9042-y